The Sturge-Weber syndrome is characterized by angiomas of the leptomeninges overlying the cerebral cortex in association with a facial nevus flammeus. Although frequently included with other neurocutaneous genodermatoses, the syndrome is almost always sporadic in occurrence. The most common associated neurologic abnormality is seizures, which are controlled in more than 50% of patients by the administration of anticonvulsants. Laser therapy is the most promising therapeutic option for cosmetic management of the facial nevus flammeus.